A subsequent proteomics study has demonstrated that complement components C3 and C9 were increased in urinary extracellular vesicles (including exosomes) derived from ADPKD patients with or without progressive CKD, whereas urinary exosomal villin-1, periplakin, and envoplakin were increased only in ADPKD patients with progressive CKD (Salih et al., 2016). This evidence concerns the gene PPL and autosomal dominant polycystic kidney disease.